Chronic granulomatous invasive fungal sinusitis (CGIFS) is a peculiar disease of the paranasal sinuses due to its rarity, patient subset, and disease course. We describe 7 cases of histopathologically confirmed CGIFS with different treatment plans and varying outcomes. Of particular note was that one of these patients developed allergic fungal rhinosinusitis after complete resolution of his primary invasive disease, a finding that has never been reported in the literature. Another patient had an atypical fungal species ( Aspergillus nidulans) on fungal stain and culture, while one immunodeficient patient had a large intracerebral disease component and died after 2 months of treatment. We also present a review of the pertinent literature investigating this rare disease.

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Sinonasal involvement was noted in approximately 30% of patients with known sarcoidosis evaluated in the otolaryngology clinic. Patients typically present with nasal obstruction and endoscopic evidence of crusting and mucosal thickening. Medical therapy with irrigations and topical/oral steroids suffices in majority of patients, with surgery for refractory symptoms being required in a small subset of cases.